OBJECTIVE: To study the clinical characteristics, treatment and outcome of childhood rhabdomyosarcoma. METHODS: The clinical data of 23 children with rhabdomyosarcoma from January, 1998 to October, 2008 were retrospectively reviewed. RESULTS: Of the 23 cases, 15 were male and 8 were female, with a mean age of 5 years old (7 months to 12 years old). Based on the American IRS staging system, 2 cases were in stage I, 4 cases in stage II, 8 cases in stage III, and 9 were in stage IV. The primary sites were found in head and neck (14 cases), extremities (4 cases), bladder (2 cases), kidney (1 case), postperitoneum (1 case) and bile duct (1 case). All of the children were confirmed with rhabdomyosarcoma by biopsy and immunohistochemistry. The clinical manifestations were related to the tumor tissues-induced space occupying, compression and erosion and were aspecific. The patients in different IRS stages were given different treatment regimens. The chemotherapy regimens VDCA, VAC or VadrC were used before 2002. After 2002, the Children′s Oncology Group (COG) protocol was employed. The two-year survival rate was 63% in 19 patients who received a combination of surgery, chemotherapy and radiotherapy, but none of 4 patients who received a surgery alone or a combination of surgery and chemotherapy or radiotherapy survived more than two years. CONCLUSIONS: The clinical manifestations of childhood rhabdomyosarcoma are not specific. A combination therapy including surgery, chemotherapy and radiation is effective to the improvement of the survival rate in children with rhabdomyosarcoma.